Sigma-Tau Rare Disease Submits Application to the European Medicines Agency (EMA) for Use of PegylatedL-Asparaginase (Oncaspar®) in the Treatment of Acute Lymphoblastic Leukaemia

July 10, 2014

Sigma Tau Pharma Ltd. (hereinafter referred to as Sigma-Tau Rare Disease), today announced the submission of an application to the European Medicines Agency (EMA) for use of its pegylatedL-asparaginase, Oncaspar® (pegaspargase) for the treatment of Acute Lymphoblastic Leukaemia (ALL) as part of a multi-agent chemotherapeutic regimen.

The submission is based on extensive clinical data generated from a sponsored clinical development program and independent investigator-initiated trials, carried on both in Europe andthe United States.

The drug is already approved in several countries including the United States, Germany, Poland, Russia, and Argentina.

With the submission to the EMA, Sigma-Tau Rare Disease aims at making the product equally available in all European Union states.

Sigma-Tau Rare Disease is dedicated to facilitating access and improving quality of care for ALL patients.

About Acute Lymphoblastic Leukaemia (ALL)

ALL is an acute form of leukaemia characterized by the growth of immature white blood cells (lymphoblasts). In patients with ALL these cells multiply to such an extent that they prevent the production of normal blood cells in the bone marrow and infiltrate other organs like the liver, spleen and central nervous system. ALL is most common in childhood with a peak incidence at 2-5 years of age, and another peak in old age.

About Pegaspargase (Oncaspar®)

The mechanism of action of Oncaspar® is based on selective killing of leukemic cells due to depletion of plasma asparagine. Normal cells, however, are less affected by the depletion due to their ability to synthesize asparagine. It is given to patients with ALL as part of a multi-agent chemotherapeutic treatment regimen.

Oncaspar® (pegaspargase) is composed of E. coli derived L-asparaginase, which is synthetically modified by covalently conjugating units of monomethoxypolyethylene glycol (PEG) to the enzyme.

About Sigma Tau Pharma Ltd.

Sigma Tau Pharma Ltd (aka Sigma-Tau Rare Disease) is part of the Sigma Tau Group, a privately owned group based in Italy. Sigma-Tau Rare Disease’s mission is to develop and make available to patients and Health Care Professionals innovative products for the treatment of Rare Orphan Diseases.

The company is based in Windsor (UK), has R&D facilities in Switzerland and the USA and markets its products through affiliates and partners in over 60 countries.